Thymic alymphoplasia.

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Abstract
Thirteen infants with thymic alymphoplasia were described. Seven were from 5 Mennonite families. The Mennonite sect is a much interbred group which immigrated to Canada from Eastern Europe around the turn of the century. The clinical, laboratory and pathological findings were compared with those in 39 previously reported cases of thymic alymphoplasia. Affected infants appeared normal at birth, but within a few days or weeks they developed respiratory and gastro-intestinal symptoms and began to lose weight. Staphylococcal, monilial and Gram-negative microorganisms were often isolated from the respiratory tract, skin, stool and urine. The condition was invariably fatal. A peripheral lymphopenia was usual, though normal or high lymphocyte counts were often found. Circulating antibodies were absent and could not be stimulated by injection of antigens. Necropsies were performed. In 12 cases the thymus was abnormally small, and in 9 thymic specimens there was a marked depletion of lymphocytes and absence of Hassall''s corpuscles. Lymphoid hypoplasia was generally prominent in lymph nodes, spleen and gastro-intestinal tract. Pulminary infection was almost invariable. In 6 cases pulminary alveolar proteinosis was indicated.